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Related Concept Videos

Structural Joints: Fibrous Joints01:03

Structural Joints: Fibrous Joints

Fibrous joints are a type of joint where the bones are connected by fibrous connective tissue. These joints provide stability and minimal to no movement between the articulating bones. There are three types of fibrous joints.
Suture
All the bones of the skull, except for the mandible, are joined to each other by a fibrous joint called a suture. The fibrous connective tissue found at a suture strongly unites the adjacent skull bones and thus helps to protect the brain and form the face. In...
Cellular Adaptation IV: Dysplasia and Metaplasia01:24

Cellular Adaptation IV: Dysplasia and Metaplasia

DysplasiaDysplasia refers to abnormal changes in the size, shape, and organization of mature cells, characterized by pleomorphism, nuclear abnormalities, and increased mitotic activity. It commonly affects epithelial tissues, including the cervix, gastrointestinal tract, respiratory mucosa, and endometrium. Although it may occur alongside hyperplasia, dysplasia is not a true adaptive response but a preneoplastic change with potential to progress to cancer.When confined above the basement...
Sutures of the Skull01:22

Sutures of the Skull

The human skull is composed of several bones that come together to protect the brain and support the structures of the face. The junctions where these bones meet are called sutures.
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
Fibrous Proteins00:55

Fibrous Proteins

Fibrous proteins are either long and narrow proteins or assemble to form long and thin structures. They contain repetitive units and usually consist of either alpha helices or beta sheets and, in rare cases, a mix of both. The amino acids in the primary structure often consist of repeating amino acid sequences. The role of fibrous proteins is primarily structural. Many are located in the extracellular matrix and are present in connective tissues to impart strength and joint mobility. They are...
Structural Joints: Cartilaginous Joints01:17

Structural Joints: Cartilaginous Joints

As the name indicates, at a cartilaginous joint, the adjacent bones are united by cartilage, a tough but flexible type of connective tissue. Unlike synovial joints, these types of joints lack a joint cavity and involve bones joined together by either hyaline cartilage or fibrocartilage.
There are two types of cartilaginous joints:
Synchondrosis
A synchondrosis ("joined by cartilage") is a cartilaginous joint where bones are connected by hyaline cartilage. Synchondrosis may be temporary or...
Growth of Cartilage and Bone Tissue01:27

Growth of Cartilage and Bone Tissue

Chondrocytes form a temporary cartilaginous model by dividing and secreting a thick gel-like extracellular matrix. Once the chondrocytes undergo programmed cell death, osteoblasts enter the site of the cartilaginous model. The process of replacing the temporary cartilaginous model with bone in an ordered manner is called endochondral ossification. In endochondral ossification, not all of the cartilage is replaced by bone tissue. Some cartilage that performs a protective and supportive function...

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Updated: May 9, 2026

Creating Rigidly Stabilized Fractures for Assessing Intramembranous Ossification, Distraction Osteogenesis, or Healing of Critical Sized Defects
07:35

Creating Rigidly Stabilized Fractures for Assessing Intramembranous Ossification, Distraction Osteogenesis, or Healing of Critical Sized Defects

Published on: April 11, 2012

Fibrous dysplasia.

Steven A Lietman1, Michael A Levine

  • 1Musculoskeletal Tumor Center, Cleveland Clinic, Cleveland, OH 44195, USA. LIETMAS@ccf.org

Pediatric Endocrinology Reviews : PER
|July 18, 2013
PubMed
Summary
This summary is machine-generated.

Fibrous dysplasia, a bone disorder, is linked to GNAS gene mutations. Targeting the Wnt/β-catenin pathway offers potential new therapies for this condition.

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Laser Capture Microdissection of Mouse Embryonic Cartilage and Bone for Gene Expression Analysis
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Laser Capture Microdissection of Mouse Embryonic Cartilage and Bone for Gene Expression Analysis

Published on: December 18, 2019

Area of Science:

  • Bone biology and developmental disorders
  • Molecular genetics and pathophysiology
  • Endocrinology and skeletal abnormalities

Background:

  • Fibrous dysplasia is a bone developmental abnormality with disorganized fibrous tissue and immature bone.
  • It can be monostotic (single lesion) or polyostotic (multiple lesions), and is sometimes part of McCune-Albright syndrome (MAS) with endocrine issues and skin macules.
  • Activating GNAS mutations are implicated in MAS and fibrous dysplasia, with GNAS acting as a gsp oncogene.

Purpose of the Study:

  • To elucidate the molecular underpinnings of fibrous dysplasia and McCune-Albright syndrome.
  • To explore the role of the Wnt/β-catenin pathway in the pathogenesis of fibrous dysplasia.
  • To identify potential therapeutic targets for fibrous dysplasia and MAS.

Main Methods:

  • Analysis of GNAS gene mutations in affected tissues.
  • Investigation of the Wnt/β-catenin signaling pathway activation in relation to GNAS mutations.
  • Review of radiographic features and current conservative surgical approaches.

Main Results:

  • Activating GNAS mutations are identified in a subset of fibrous dysplasia and MAS cases.
  • Gas mutations were shown to activate Wnt/β-catenin signaling.
  • Classic radiographic findings include a ground-glass appearance due to defective mineralization.

Conclusions:

  • Fibrous dysplasia has a known molecular etiology involving GNAS gene mutations.
  • Wnt/β-catenin pathway inhibition presents a promising therapeutic strategy.
  • Further research into molecular pathology may lead to improved conservative treatments for fibrous dysplasia and MAS.